Carglumic acid is an orphan drug used for the treatment of hyperammonaemia in patients with N-acetylglutamate synthase deficiency. This rare genetic disorder results in elevated blood levels of ammonia, which can eventually cross the blood-brain barrier and cause neurologic problems, cerebral edema, coma, and death.

Nicotinuric acid is an acyl glycine which is normally minor metabolites of fatty acids. Acyl glycines are produced through the action of glycine N-acyltransferase.

Schisphenlignan I.